Hepatopulmonary Syndrome - Illness Script HPS is a pulmonary disorder of chronic liver disease characterized by a triad of: 1) chronic liver disease 2) pulmonary vascular dilatation & 3) gas exchange abnormalities PATHOPHYSIOLOGY: • Intrapulmonary capillary vasodilation leads to impaired arterial oxygenation through V/Q mismatch • Mismatch? Dilated vessels + absent tone = increased flow & longer distance for O2 to cross // overperfusion + decreased RBC transit time = increased Aa gradient • Intrapulmonary arteriovenous shunting causes mixed blood to pass through pleural and pulmonary a/v communications // a higher presence in the lower lung zones may be behind the mechanism of orthodeoxia CLINICAL FEATURES: • Progressive dyspnea • Platypnea - worsening dyspnea when moving from supiine to upright • Orthodeoxia - decrease in PaO ≥ 5% (or 4 mmHg) when moving from supine to upright • Stigmata of cirrhosis: jaundice, ascites, palmar erythema, spider angiomas, digital clubbing DIAGNOSIS - TRIAD: • Liver disease &/or portal HTN • Aa gradient ≥15 mmHg (room air) - ABG • Pulmonary vascular dilatations - contrast-enhanced TTE with agitated saline - Microbubbles elude capture and reach the left heart TREATMENT • No correlation between the presence or severity of HPS and the severity of liver disease (CPT. PELD. MELD) • In one large study, HPS mortality rate of ~12% • No effective medical therapies other than supplemental oxygen • Liver transplant is the only definitive treatment. Marked improvement or resolution of syndrome in 85% post-transplant By @Alert_Oriented3 #HPS #Hepatopulmonary #Syndrome #cirrhosis #diagnosis #hepatology
